Inflammatory pseudotumor-like follicular dendritic cell sarcoma mimicking a colonic polyp

Zhao, Min; Du, Xun; Ouyang, Bowen; Li, Mingxian; Yang, Haifeng

doi:10.1007/s11605-021-04961-y

Cited by 5 publications

(2 citation statements)

References 3 publications

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“…After an extensive survey of the English literature, we found 70 reported cases of EBV + IFDCS 2–8,12–34. The detailed clinical data of the 70 cases from the literature are included in Table S2 (Supplemental Digital Content 2, http://links.lww.com/PAS/B509).…”

Section: Resultsmentioning

confidence: 99%

Challenges in the Diagnosis of Epstein-Barr Virus-positive Inflammatory Follicular Dendritic Cell Sarcoma

Yang

Tao

et al. 2022

American Journal of Surgical Pathology

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Epstein-Barr virus (EBV)-positive inflammatory follicular dendritic cell (FDC) sarcoma (EBV+ IFDCS) is a rare entity, and its histopathological characteristics have not been fully described. Here, we investigated the wide morphologic spectrum and immunophenotype of this tumor with the aim to help avoid misdiagnosis. Thirteen cases of EBV+ IFDCS were retrospectively analyzed, combined with a review of 70 cases reported in the literature. The median age of patients was 49 (range, 29 to 67 y). Six patients were male and 7 were female. Most cases (92.3%, 12/13) occurred in the liver or spleen, and only 1 case affected an extra-hepatosplenic site (lung, 7.7%, 1/13). Tumors were assessed for a variety of histologic features and assigned to the following morphologic groups: classic type (53.8%, 7/13), lymphoma-like subtype (38.5%, 5/13), and hemangioma-like subtype (7.7%, 1/13). The classic type had distinct EBV-positive neoplastic cells with a fascicular or storiform growth pattern, variable lymphoplasmacytic infiltrates, and blood vessels. The lymphoma-like subtype had extremely prominent lymphoplasmacytic infiltrates (resembling marginal zone lymphoma with plasmacytoid differentiation) with singly dispersed distinct EBV-positive neoplastic cells, highlighted by in situ hybridization for EBV-encoded small RNA. The hemangioma-like subtype had extremely prominent blood vessels with hyaline and/or fibrinoid degeneration, singly dispersed distinct EBV-positive neoplastic cells, and limited lymphoplasmacytic infiltrates. Immunohistochemically, the neoplastic cells showed variable staining for FDC markers (CD21, CD35, CD23, and SSTR2) and the fibroblastic marker SMA, with the staining ranging from very focal to extensive. The number of EBV-positive neoplastic cells ranged from 80 to 400/HPF. All cases showed variable expression of PD-ligand 1 (PD-L1) (CPS: 5-90). IgG4-positive cells ranged from rare up to 100/HPF. Interestingly, 2 cases satisfied the criteria proposed in a previous study, mimicking IgG4-related disease. EBV+ IFDCS is an entity with an extremely wide morphologic spectrum and immunophenotype. Awareness of the spectrum of morphologic presentations of this rare tumor, specifically the lymphoma-like subtype and hemangioma-like subtype, is important for accurate diagnosis.

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Section: Resultsmentioning

confidence: 99%

Challenges in the Diagnosis of Epstein-Barr Virus-positive Inflammatory Follicular Dendritic Cell Sarcoma

Yang

Tao

et al. 2022

American Journal of Surgical Pathology

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“…The clinical and pathological characteristics of EBV+ iFDCS presenting as a colon polyp, including the current case, are summarized in Table 1 . There have been only 12 cases reported in the literature thus far [ 5 , 8 , 9 , 10 , 11 , 12 ]. The patients’ ages ranged from 46 to 78 years, and the mean and median were 58.8 and 56 years, respectively, without any evident sex predominance (female: male = 7:6).…”

Section: Discussionmentioning

confidence: 99%

Epstein-Barr Virus-Positive Inflammatory Follicular Dendritic Cell Sarcoma Presenting as a Colonic Polyp: Report of a Case with a Literature Review

Huang

et al. 2023

Medicina

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Background: Follicular dendritic cell (FDC) sarcoma is an uncommon mesenchymal origin neoplasm derived from the abnormal proliferation and differentiation of FDCs. Epstein‒Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+ iFDCS), which used to be known as the inflammatory pseudotumour (IPT)-like variant, occurs exclusively in the liver and spleen and has rarely been reported in the gastrointestinal tract. Case study: Here, we report a case of a 52-year-old woman with a special family history undergoing a routine physical examination. The colonoscope revealed an approximately 18 mm transverse colonic polyp, and the endoscopic polypectomy was performed. Microscopically, the excised polypoid mass was composed predominantly of inflammatory cells scattered with atypical ovoid to spindle tumor cells. Interestingly, there was a remarkable infiltration of IgG4+ cells. Immunohistochemistry showed that the tumor cells were positive for CD21, CD23 and CD35. EBV-encoded mRNA (EBER) in situ hybridization also gave positive signals. These histopathology features supported the diagnosis of EBV+ iFDCS. The patient was free of disease over 1-year follow-up. Conclusion: Identification of the potential pathogenesis sites of EBV+ iFDCS in extra-hepatosplenic regions is necessary for correct and timely diagnosis, and we consider it very meaningful to share our experience of diagnosing this tumor type. Furthermore, we summarize the clinicopathological features of EBV+ iFDCS presenting as a colon polyp after a thorough review of the literature.

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Clinicopathological and Imaging Characteristics of EBV Positive Inflammatory Follicular Dendritic Cell Sarcoma

Zhao,

Wang,

Liu

et al. 2024

Preprint

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Objective EBV-positive inflammatory follicular dendritic cell sarcoma (EBV + inflammatory FDCS) is a rare tumor that has been less systematically studied. This study aims to improve the understanding of EBV + inflammatory FDCS by analyzing radiological imaging data and clinicopathological features. Methods A retrospective analysis was performed on twelve patients with EBV + inflammatory FDCS confirmed pathologically. Ten patients underwent computed tomography (CT) scans, six underwent magnetic resonance imaging (MRI) scans, and four patients underwent CT and MRI scans simultaneously. Results Twelve patients (six in the liver and six in the spleen) had clinically asymptomatic or asymptomatic, and six cases were found on physical examination. Pathologically, the tumor cells dispersed in a background of inflammatory cells. All cases showed positive expression of at least one follicular dendritic cell (FDC) marker and EBV. On unenhanced CT images, nine cases of EBV + inflammatory FDCS presented as well-defined iso-or hypodense masses, and one liver case had an irregularly shaped lesion with unclear borders. All cases showed iso-or hypodensity signals on T1WI and mixed signals on T2WI. The hepatic lesions showed marked enhancement in arterial phase and diminished enhancement in portal vein phase and delayed phase, whereas the splenic lesions showed mild enhancement in arterial phase and continued enhancement in portal vein phase and delayed phase . Conclusion EBV + inflammatory FDCS is a rarely low-grade malignant tumor, which exists the possibility of local lymph node metastasis and recurrence. Therefore, the preoperative diagnosis is expected to improve by carefully analyzing the imaging features and clinical characteristics of the patients.

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Inflammatory pseudotumor-like follicular dendritic cell sarcoma mimicking a colonic polyp

Cited by 5 publications

References 3 publications

Challenges in the Diagnosis of Epstein-Barr Virus-positive Inflammatory Follicular Dendritic Cell Sarcoma

Challenges in the Diagnosis of Epstein-Barr Virus-positive Inflammatory Follicular Dendritic Cell Sarcoma

Epstein-Barr Virus-Positive Inflammatory Follicular Dendritic Cell Sarcoma Presenting as a Colonic Polyp: Report of a Case with a Literature Review

Clinicopathological and Imaging Characteristics of EBV Positive Inflammatory Follicular Dendritic Cell Sarcoma

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